A 50-year-old man was referred for dizziness, bradycardia and dysphagia .He was very clear in describing his symptoms and landed up in Gastro- enterology OPD , from there was referred to my clinic for cardiac work up . His ECG showed a sinus bradycardia HR of 48 /mt.

Echocardiogram revealed a structurally normal heart as we expected , but was surprised to spot suspicious shadow in para-sternal long axis view , beneath left atrium.

A well demarcated large mass compressing left atrium. Trans Thoracic Echocardiography may not be looking at the heart alone ,(Its technically Thoracic Ultrasound though we may refer it as Echocardiogram )

Aortic aneurysm ?

Mediastinal teratoma?

Bronchial adenoma ?

Esophageal mass ?

The Answer is none of the above

As I was wondering what it was, the staff nurse in charge threw a heavy folder with well worked up gastro Investigations.

That moment , diagnosis became obvious , without a need for further scrutiny to my medical acumen.

Note: The barium swallow of the Esophagus reveals the Intimate relationship between the food tube and the heart as it descends vertically downwards posteriorly . Realise , how the proximity of these two structures could confuse a physician when symptoms spill over on either way. (I would have expected a lateral view to show the compressive effect of Esophagus on the left atrium the radiologists felt its not important !)

Yes , it is Achalasia of the cardia , dilating the lower end of esophagus with fluid /mass effect , compressing the posterior surface of Left atrium.He underwent a myomectomy surgery.

Achalasia cardia is known to be associated with symptomatic bradycardia, dizziness, and rarely swallow syncope,though this patient didn’t have a classical syncope.The bradycardia is probably due to high vagotonia, (Hugging effect on posterior surface of heart known for rich innervation of vagus.) . Complete reversal of bradycardia after esophago -gastric surgery is expected.

Implication for cardiologists

There has been instances of patients with esophageal syncope and reflex bradycardia getting permanent pacemaker therapy. I think , clinical or sub clinical esophageal disorders should be included in the work bradycardia before labelling them as intrinsic sinus node dysfunction .(Ref 1,4)

Final message

The field of Cardiology is often referred to as a super specialty atleast in India . I disagree with it strongly. Cardiologists are neither super(eme) nor special .We need to be reminded its afterall a sub-specialty of Internal medicine and each specialist should undergo retro-training in medicine periodically .This patient is a typical example of a gastric problem entering the domain of cardiac Imaging.Strong foundations in symptom analysis and some degree of medical curiosity will enable an occasional cardiologist to make a correct diagnosis belonging to a remote foreign specialty.

Hey , What’s that moving object over AML ? It looks odd, it doesn’t look like a thrombus or a vegetation.

Yes, I agree , its moving independently but I think , Its benign threads of fibrin attached to the valve .They are called as valvular strands.

Is it ?, I haven’t heard about it ! Can you please tell me something about it.

Strands are highly mobile, fine, filiform threadlike excrescences that is seen arising from valvular structures. Synonym : Its same as Lambl’s excresceneces , the Czech physician who described it over Aortic valve in 1860.

The following TEE clip shows strands attached to Aortic valve

Incidence

Reported Incidence of valvular strands varies .Some reports suggested it may be up to 5-10 % .( SPARC study Mayo clinic 1999 its staggering 46 % !)The reason for such high incidence is, many of us are still not clear what we refer to as strand.The imaging modality also has a say. With improving resolution of TTE and liberal TEE use more strands are detected .A recent large study from Israel , suggest a good news , in large population based study (21,000) true strands are observed in just around 1 %.(MarinaLeitman 2014 )

Is it Physiological or Pathological ?

The valve closure lines are physiologically stressed , some amount of denudation of endothelium is expected .This leads to a thrombus formation along with the exposed mucopolysacchride layers of the valve form a filiform ,filamentous structure. .To call it physiological or pathological is left to our wisdom and perception. The size however matters. It could be the reason behind many unexplained strokes.

What is the natural history of these strands ?

Its difficult to believe It may persist for lifetime.If its truely fibrinous strands it may have a life cycle and disappear.

Size

Should be less than 1 mm.

Length varies between 3 mm to 5 mm

Location

Can be seen in any valve or even in aortic root.

Attachment : Atrial side of mitral valve and ventricular side of Aortic valve.

Strands over prosthetic valve is also reported.

Clinical significance

It has three common issues.

One: Getting confused with other more pathological entities.

Two : Risk of stroke.

Three: Nidus for normal native valve endocarditis ?

Strands may closely mimic

Vegetations

Bland thrombus

Redundant leaflet /Chrodae (Marfan and variants)

Disrupted chordae (After MVR)

Flail leaflet

Fibroelastomas

Risk of dislodgement and stroke

These strands are minute. It seems plausible dislodgement need not necessarily result in stroke or other organ ischemia.We don’t know whether it gets dissolved on transit.However the risk of stroke is increased in most reports except few studies(Roldan).

Management

First question to ask is , Should we inform our patients about these ubiquitous accessory valve tissues if detected incidentally ?

Largely benign and can be ignored in most.A follow up echo may be adviced once in a year or 2. (I have one anxious patient after I reported such strands in Marfan syndrome )

In patients who has h/o stroke presence of these strands gains importance and is an indication for anticoagulation.

Surgical excision of large strand is a dramatic option and is rarely performed.

Queries with no answers

Is it accessory valvular (mesenchymal) tissue ?

Does Atheromatous plaque contribute to these strands in Aortic valve ?

Strands , if disappears by natural means , do they regrow from the same spot of raw surface ?

Final message

Fibrous strands detected over the valves by routine echo are uncommon .However , It may give considerable anxiety if documented and reported to our patients and physicians .Though these have negligible clinical significance , the risk of stroke is increased in those with large strands.

RV when exposed to sudden pressure overload it not only dilates , it’s wall stress increases (Laplace law : Wall tension = P x Radius ) and end up mechanically stunned . But , since the RV has a complex shape the distribution of this stress is not uniform .As the RV assumes more spherical shape the apical part is not exposed to this stress as it tend to abut under LV.

RV apex is anatomically tethered with LV apex and share significant amount of circumferential fibres .In patients with acute pulmonary embolism , LV usually is hyperkinteic due to tachycardia .This pulls the RV apex along with it for a proxy contraction .

Rarely , primary RV ischemia due to RCA under perfusion* may be responsible for this unique wall motion defect . Since RV apex is mostly supplied by LAD it is free from ischemia . (*Acute elevation of RV intramural pressure due to PHT , compromising RCA perfusion pressure )

While 2D echo visualizes the LSVC , it is the color Doppler flow (in blue ) that confirms the flow going away from transducer towards coronary sinus .Please note , if the LSVC shows red flow it indicates the left vertical vein and the flow is from below up .This is supra cardiac TAPVC . It makes immense embryological sense to understand LSVC and and left vertical vein are both same entities only the connections are different .

Anterior mitral leaflet (AML) is an unique structure in the heart .It is the fastest moving structure inside the heart . It is the first structure visualised by echocardiogram by Elder and Hertz in early 1950s .

While AML is known for vigorous motion , the PML motion is subdued . By tradition AML shows a motion which resembles alphabet M .

But AML is not be taken lightly . It can change it’s motion not only in pathological states but also in health . One such pattern is trifid motion of AML . Following is a Echo Image in a perfectly normal Individual .

Possible mechanisms underlying Trifid motion of AML

The plane of M-mode cut will change the mitral valve motion .(May be this is most common ).M-mode at tip of mitral valve may be trifid ,however a little beyond may record a bifid-M pattern .

Redundant mitral valve

Mid diastolic AML drag

Signs of elevated LVEDP

Finally , it could be a sign of mitral valve fatigue after excrcise . Some of these persons revert back to M pattern after a brief period of Trifid motion following exercise .

Does trifid AML motion result in Tri-phasic doppler flow as well ?

Mitral valve filling is classical E and A .

This usually correspond to M pattern of anatomical AML motion .

Do the anatomy goes hand in hand with physiology ? Will the mid diastolic AML drag result in augmented flow ?